Pos0889 case Series of Patients With Eosinophilic Fasciitis Secondary to Immune Checkpoint Inhibitors Therapy

Abstract

BackgroundEosinophilic fasciitis (EF), a fibrotic disease that causes inflammatory infiltration in the subcutaneous fascia is clinically characterized by edema and subsequent induration and tightening of the skin and subcutaneous tissue. EF is a rare immune-related adverse event of immune checkpoint inhibitors (ICI).ObjectivesThis article aims to investigate the clinical features of ICI-related EF and to improve the understanding of the disease among rheumatologists.MethodsSearching relevant articles in the electronic databases Medline, PubMed, Science Citation Index, China Biomedical Literature Database (CBM), China Journal Full Text Database (CNKI), and WANFANG Data with the key words of “eosinophilic fasciitis” or “Shulman syndrome” and “checkpoint inhibitor”, “CTLA-4”, “PD-1” or “PD-L1”. Only articles or case reports with detailed medical records of ICI-related EF patients were included. ICI-related EF patients in our department were also included.ResultsA 58-year-old male patient with angioimmunoblastic T cell lymphoma presented with skin edema, subsequent induration, tightening of the skin, and subcutaneous tissue (Figure 1 A&B). The eosinophils elevated in peripheral blood after 6-month treatment of PD-1 inhibitor (Camrelizumab). The patient was diagnosed as ICI-related EF and then was referred to our rheumatology department. Other seventeen EF patients from the above databases were also included for analysis. Among these 18 patients, 50% (9/18) were male and the mean age was 57±14 years. The most commonly used ICI was PD-1 inhibitor such as nivolumab and pembrolizumab, accounting for 72% (13/18), next was PD-L1 inhibitor (17%, 3/18), and 3 patients used PD-L1 inhibitor combined with CTLA-4 inhibitor. Of all cases, 50% (9/18) was metastatic melanoma and 17% (3/18) was lung cancer. After ICI treatment, the median onset time of EF was 12 months. The most common involved organ was skin and 94% (17/18) of patients presenting with symmetrical skin edema and sclerosis. For other skin manifestations, 44% (8/18) of patients had typical “groove sign”, and one case showed unilateral skin involvement of skin tension and erythema from pubis to left anterior iliac crest. When the affected limb raised, there is a visible sulcus due to the decrease of venous dilation pressure, namely “sulcus sign”. The second common involved organ was the joints which were presented with limited function in 56% (10/18) of patients. Additionally, 39% (7/18) showed muscle involvement such as myalgia and myasthenia. Increased eosinophils in peripheral blood was observed in 72% (13/18) of patients. Twelve patients (63%) received MRI examinations which showed the signal in the subcutaneous and deep fascia increased in both fat-suppressed T2 images(Figure 1 C&E) and post-enhancement T1 images(Figure 1 D&F). Fifteen patients underwent full-thickness skin biopsy at the lesion site, the pathological changes in all patients supported the diagnosis of EF. The ICI was discontinued in 94% of patients. Meanwhile, 83% received glucocorticoids and 56% of patients were treated with methotrexate. After these treatment, the clinical symptoms of EF improved in 89% of patients (n=16), while eosinophils returned to normal after a median treatment time of 2.5 months. EF progressed even through the combined treatment of prednisone, MTX, and abatacept in one patient. Another one case died after 6 months due to metastasis of bladder cancer (stageIV).ConclusionAlthough EF is a rare adverse effect of ICI treatment, individuals receiving these drugs should be monitored closely for symptoms of EF. Discontinuation of ICI and administration of immunosuppressants may prevent the progression of EF.Disclosure of InterestsNone declared